So Long TPN – Don’t let the door hit you on the way out!

First the good: Today is Isaak’s last day on TPN! His Broviac catheter will be left in place for another month in case we have to backtrack and put him back on some sort of IV, but we are thinking positively on this one.  He’s done very well on the low volume over the last week. Until removal, we will only have to flush the line daily and change the clave/connector twice a week

In news almost as exciting, he will now have a 4 hour gap in continuous feeds. He’s getting the same volume through his g-tube but over 20 hours instead of 24. This means we have 4 hours of a cordless baby! This is going to be a good thing for Isaak and mommy and daddy.

Both of the little guys continue to grow at an astounding rate. On Monday, Isaak weighed in at 14lbs even and Penny at 15 lbs 4oz. Amazing. Isaak has really taken off since they began allowing us to feed him on demand and Penny just keeps on trucking. She is such a chubby monkey now with little leg rolls and arm rolls and the biggest cheeks you have ever seen. We’ll have to get some diaper only pictures soon for the world to see ;)

Now the not so good: Isaak had a physical therapy evaluation on Monday. They were very happy with the neuromuscular side of things and he seems to be developing fine motor control reasonably well (such as passing an object from hand to hand) – BUT – his gross motor skills are lagging. That would include head control and attempting to sit up or roll over. We have certainly noticed a gap compared to his sister and mentioned as much during the SICC visit. As such, we’ll be looking at getting weekly PT visits to help him along as well as focusing our attention on helping him work towards goals.

We think that some of this may be because he just hasn’t had the same opportunities as his sister. He’s been hooked up to IVs and feeding tubes so much that our interaction has been more limited and we’ve been unable to hold him or place him in certain positions. Now that he is cordless for a few hours it will be much easier to work with him. We have no idea at this point if this anything other than developmental lag. We really hope so.

Now the downright bad: Isaak had an eye exam last Thursday and the results were not encouraging for his left eye.  When last we visited Duke Eye Center, we thought everything was going to be pretty stable but the doctors let us know that nothing was predictable in these situations. It now appears that additional fibrous tissue is growing around the retina and is causing more tugging, greatly increasing the risk of full detachment.

We may have reached a point where surgical intervention will be required to try to preserve the vision he has in that eye. On Friday (12/17), he will be undergoing a detailed exam under anesthesia, and depending on the results, they will progress immediately to surgery. If surgery is necessary, odds are good that he will lose the lens in his left eye and will have to wear a contact lens to protect it and allow proper focusing. 

The left has been of great concern since a massive hemorrhage back in the summer. It appeared that it had cleared up and stabilized but apparently damage was caused that might have been behind the retina and this has led to the continued problems. No question this is a big disappointment. Little guy has seen enough of OR for a lifetime, much less for a baby not even 8 months old yet.

On the plus side, his right eye is doing really well and is essentially mature. 

That's it for now (quite a lot, really) and we'll have more information on Friday regarding surgery (or hopefully lack thereof).

Just in Case You Were Wondering...

...Isaak is, in fact, at home and doing well. His stay on the pediatric floor at Duke was just a formality and he was discharged after one night with no fanfare (though he did make friends with the nurses).

We really won't have an update on the situation with his eyes until Monday morning. He has a follow up appointment at Duke Eye Center with the surgeons.

Otherwise, everybody is doing pretty well. The last official weights we had on these two, Penny was just shy of 11 and 1/2 pounds (I bet she is closer to 12 and 1/2 now) and Isaak was just a tick over 10. So "Little Bit", as I had taken to calling her, really isn't anymore. She appears to be taking full advantage of that intact digestive tract she is sporting to pack on the pounds and inches. Isaak is jealous. Honestly though, if we didn't have Penny to compare him to, I think his growth would look great to us and according to the doctors, his growth really is quite good given his disadvantages.

He had a visit with the GI Docs, GI Surgeons and Speech Therapist at UNC last Wednesday and everyone was pleased with how well he's doing. Amazed really. The surgeons increased the size of his G-tube (since he was a tiny tyke that didn't even weigh 5 lbs when he had his reanastomosis) and were otherwise happy with the way everything looked.

The GI docs have been very happy with his feeding progress and he's now getting 5, 45ml (1.5oz) bottles a day. This is quite an increase from the 3x10ml he was getting when he came home. He's still getting TPN via his Broviac but there is hope we can start tapering that down soon since he's doing so well with his g-tube and oral feeds. They have decreased the amount of dextrose in his TPN and that, combined with the oral feeds, has been great for his liver. His total bilirubin is down to 5.3 compared to 11.6 not long after he came home from the hospital. Remarkably, he barely even looks jaundiced now. Ideally, the bili level would be below 1.2 mg/DL, but we'll take what we are getting now, yes sir!

The speech therapist was making sure he was doing OK with his feeds. When we first began increasing bottle feeds, he was having some trouble developing suction. Apparently, that was just due to inexperience and he is now doing a great job and they did not have any concerns.

I took my first extended trip out of town since last spring...and nothing terrible happened. Things like that really ride in the back of your mind. Grandma Karla is in town too, so that made it a little easier on everyone. I wouldn't have taken off if Bekka was by herself that entire time.

Look for an update on Isaak's eyes later this week and new photos by the end of the month. I'm going to start  uploading photos about once a month just so I'm not chasing my tail all the time trying to keep up.

This Post Just Couldn't Weight

Yes, the pun is horrible and completely intended. That's just how I roll.

In big news, Isaak was weighed today: 8lb 12oz
Penny was weighed today: 8lb 12oz

Technically, he weighed 10 grams more (3970g vs. 3960g) but Penelope has caught up with and is quite likely to soon pass her brother in weight. Isaak has been bigger than Little Bit since they were born! She was 1lb 7oz and he was 1lb 11oz (780g vs. 650g), and he has typically maintained an 8oz margin for months.

So why the sudden overtaking maneuver on Penny's part? Simple: She eats like an NFL linebacker and still has her entire digestive track, whereas Isaak is getting about 30% of his nutrition via TPN and the rest via g-tube and three small bottles a day. She can just consume, and more importantly, process more calories than her younger brother.

What is amazing is that Penny isn't chubby at all. She has a tiny double chin and a few small rolls on her legs, but is mostly solid muscle. Apparently all her food consumption is going right into getting bigger. Isaak is definitely the chubbier of the pair and looks really healthy - he just isn't growing as fast. They will both get measured at the pediatricians office on Wednesday and it will be interesting to see how much longer she is.

Please don't take this development as a negative. Overall, Isaak is doing so well! He's gaining at a nice steady clip of 0.5 to 1oz per day and has tolerated every feed increase we've tried since coming home. In fact, right now he is at 16ml/hr - the rate that caused him to start dumping while we were in the NICU. Of course, he also weighs probably 3 lbs more now than he did then and should have had a commensurate increase in digestive tract length and development.

In very good news, we have been allowed to start decreasing TPN as his g-tube feeds increase. Hopefully we'll start to see a drop in bilirubin levels as the ratio of oral/enteral feeds to TPN increases even further.  To stop TPN, he would need to be at about 25ml/hr of enteral feeds. Of course, as he grows and gains weight, that target moves out a bit, but we are optimistic we can get rid of TPN in a reasonable time frame. Truly, from what we've found in our research, he's doing remarkably well for a short-gut baby.

He has a long road ahead but is on the march like a little soldier.

Sound Medical Advice (and an update on the Twins)

First, to get this out of the way:

So now you know! And catheter safety is paramount (really).

One of the biggest risks with a central line is infection and another risk is some sort of trauma caused by tugging or moving the catheter. But just wow! I suppose these videos are geared for kids and their parents and not so much little ones, but now Bekka and I can better resist the temptation to start jumping rope once we get him home.

Now on to the babies:

Isaak is doing pretty darn well. Some of you may have been updated via other means that Isaak was moved to a regular pediatric room last Wednesday. The way it happened was...interesting. We'd been debating making the move for a few days, a new attending came on in the NICU and pretty quickly decided that Isaak really should go to the floor. He no longer has preemie problems (save for a bit of anemia) and the doc had decided that the GI folks could better handle the feed situation. We did not disagree.

Anyway, on Wednesday, Bekka decided to take a day off from the environs of the NICU as she knew Isaak would be well cared for by all the nurses. She'd been told it would be "2 or 3 days" before a room would open up on the regular pediatric floor. That was the status at 3pm on 8/11. At 6, she gets a call that says "we will be moving him in a hour". So not only do we get to hurry to the hospital, we have to pack for Bekka to spend some time there. So we did. We finally made it to the hospital to find him zonked out in the bed and sucking merrily on his paci. That was a hectic evening.

Ever since, he's been ensconced on the 6th floor of the North Carolina Children's Hospital under the watchful eyes of his mommy. Little sister has been there with them every night save one, when daddy and Nana and Papaw brought her home so mommy could sleep without having baby grunting in stereo running all night long. (Apparently it's tiring. Who knew?)

Medically he seems to be rapidly improving since Friday. He was on 10ml per hour via g-tube for a week as they monitored the level of "reducing substance" in his stool. (Poop. You KNEW there was going to be poop, right?). This test measures the body's ability to absorb sugar in the intestinal tract and is an important indicator of bowel function. For several days, he was running high values (0.5 or greater g/dL). Yesterday, he finally dropped down to 0.25. Now, this doesn't mean that he wasn't processing fats and proteins, just sugars but it's a good test looking at how things are going. Since he hit 0.25 for 2 days in a row, on Monday they upped him to 12ml/hr of 50% milk and 50% Elecare (an elemental formula). If he has a good test tomorrow, he will likely get bumped to 13. They left his TPN where it is so he could get a few more fluids.

He's gaining weight well at roughly an ounce a day, and that's a good indicator he is also getting nutrition from the g-tube feeds. If he wasn't absorbing something, the TPN doesn't have enough calories to keep him growing. Do we think he'll be off TPN before coming home? Probably not. We are at the point though where we'd like to get our little guy to the home and used to be being in a non-hospital environment. He would likely be looking at many months before getting to strictly oral feeds, but you never know. The next big step for him will be trying small bolus feeds, both via g-tube and orally. Ideally, when he comes home, we'd be able to feed him orally during the day and then have g-tube feeds overnight.

His poop is looking better, too. A little color has started showing up in it and that corresponds with a sudden change in his overall color. He's still jaundiced, but the change from Friday to Saturday was pretty stunning. Apparently, much of the color in poop is the body ridding itself of bilirubin (I did not know that) and we hope that his body has decided to get better at doing just that. The TPN is still not good for his liver, but he'll be so much better off if he can dump the bili.

In other news, Isaak's edema seems to have completely disappeared. Much like Penny, he just started peeing a couple of weeks ago and hasn't stopped. This is excellent since he has the IV in all the time. He also appears to be working through the anemia (At last! We hope.) His last hematocrit was only 0.1 lower than the previous (so effectively the same). Hopefully his next will trend higher. Also, his eye exams are starting to look gradually better. The left eye is still the most concerning, partly because there is a hemorrhage concealing the retina in a couple of places. Hopefully that clears up more this week so they can confirm that the eye is still maturing.

Penny has been her usual rambunctious self. We'd like her to be gaining weight a bit faster, so we have just upped the quantity she is getting in her bottles to almost 3 oz. She gets 3 of these a day, all fortified with formula to 24 calorie. Bekka also reports that she is getting better and better at nursing. We have to remind ourselves that these little guys have to practice and get stronger and that they were only due last week! Her only other issue is a worsening of the RoP in her right eye. It's a little unusual for that to be happening at this age and stage of development so she'll be back next week for another checkup. Hopefully nothing to worry about but it's important to stay on top of that issue.

I think Bekka and I are mostly staying sane. She's spending all her time at the hospital save for Penny's appointments and when I spirit her away to escape for a bit. It's very challenging taking care of two babies but there is something to be said for not having to drive back and forth to the hospital every day and getting to stay in the same space at night.

In a complete aside, did you know that at the state's preeminent public hospital (PUBLIC), patients have to pay $8 a day to park. WakeMed was FREE for patients and Duke was only $2 a day when we were there with Ollie. Just amazing. They have their excuses and they all suck. I can only imagine how many patients can't actually afford that. Serving the state well there Carolina...

There is a reason for cautious optimism in our posts! Also: Warning, poop ahead!

So, the day *after* the big update regarding Isaak and all his good news, he decides to have the predicted setback in his feeding progress. When we were visiting on Sunday, he had a little spit up (completely new event) and also a big poopy diaper that was much looser than they had been. (Sorry, poop is the topic of conversation of lots of baby parents, but especially ours ;)

On Monday, he had an even bigger diaper that was definitely too liquid. Lab analysis indicated that he was not processing carbohydrates well at all. This is where you have to step back, retrench and re-evaluate.

The docs immediately dropped his oral feeds down to 10ml/hr (from 16). He will resume TPN (grrr) this evening to make up for the missing calories (he was on dextrose and electrolytes overnight). We hate to see the resumption of TPN, but it's critical he get the calories to continue growing and developing in other ways right now.

The good news? He hasn't pooped since yesterday evening and this probably means the drop in volume really has helped him slow down his food transit time. Sometime in the next couple of days, they will run labs on his stool again  to make sure everything is OK and he is processing well. Also, his electrolytes and glucose still looked good. If a baby is truly "dumping", those usually get out of whack really fast. It's great that he's still able to get a fairly substantial amount of enteral feeds as that is so so important to intestinal development and growth.

Over the next few days, they will begin increasing his feeds again and we'll see how far he gets this time. I suspect things will go a little bit slower and more cautiously. We absolutely want to maximize the amount of enteral feeding he gets so we can minimize TPN, but we also have to keep him growing and developing. There is a good chance they will also begin increasing the percentage of his feeds that are based on an elemental formula (say 50/50 formula and breast milk) as those are often more readily absorbed.

That's it for now.

Isaak's big Update

I'm going to try to make this relatively short. Isaak is doing great this week. Fantastic even.

Early in the week they made another effort to get him off supplemental oxygen, and so far, 5 days later, he's been a little champ.

First off, new pictures: Here and There

Now on to the update.

Here's a bulleted list of Isaak info:

• Now weighs 7lbs 5oz.
• Off oxygen
• Still anemic but his reticulocyte count is improving. There is still a chance he will need a transfusion again and they'll be checking his blood-work again in a couple of days. Hopefully he's bottomed out on the anemia and will show signs of improvement. This makes the fact he's not using oxygen all the more remarkable.
• Off IV nutrition (TPN) for now. He's tolerating g-tube feeds really well and if he gets up a couple of more notches, there is a chance he will not need to go back on TPN.
• RoP has improved quite a bit. Ophthalmologist was optimistic after the exam on Friday. His eyes have begun maturing and the hemorrhages that were present have begun shrinking. By no means is he out of the woods, but it surely is looking better (no pun intended there).
• We might move him to a regular pediatric ward. He doesn't really have any preemie problems right now (apneas and bradys) and Bekka could room-in with him and bring Penny along. Of course, there is a big safety net in the NICU if something suddenly occurs, so it's not an easy decision.
• Moving to a regular floor was definitely the plan when we were positive he was coming home on TPN. He would need to be on GI service so we could get TPN training and get his mixture calibrated. Staying there may still help the GI folks assist us in getting him used to bolus feeding (and he might also need TPN if he has any problems over the next few days).

If he really doesn't come home with TPN (double yay!), he will hopefully be getting bolus feeds (bottles and nursing) during the day and then continuous feeds overnight. This will help make sure his system isn't overwhelmed and that he gets enough calories and nutrients. Again, a lot is still up in the air. He is doing significantly better than anyone expected at this point and some small setbacks are to be expected but he is a tough little hombre.

Not that much to report on Penny. She continues to light up her parents' days and is now up to a whopping 6lbs 4oz. Tiny, but much bigger than she was. Big enough for a regular car seat even! We don't have length measurements on either of these little tykes. We'll try to get those soon. She's a spunky little girl (hmm, wonder where that comes from?) and seems to have inherited her mom's sleeping patterns.

Surgery Day is Here

Today is Isaak's big day. The little fella is scheduled for 3 surgical procedures.

1. Reanastomosis of his upper and lower intestines and repair of his ostomy
2. A Broviac Catheter (smaller version of a Hickman Line).
3. Placement of a g-tube, likely with a Mic-Key button

Yes, it's a lot for such a tiny guy to go through but there is a reason for it all.

The reanastomosis is obviously the big one. An upper and lower GI contrast dye study was conducted today and the results were encouraging. Isaak had no visible strictures. Now the big question is will everything be healthy when they open him up. They may have to do small resections at the ends of the remaining intestinal tissue to make sure they are connecting the healthiest, most viable tissue back together.

The Broviac Catheter will allow the continuation of parenteral nutrition and is typically easier to maintain, longer lasting and with fewer infections than the PICC lines they have been using so far. It is almost certain he will need to continue parenteral nutrition for weeks to months to help him continue to grow while his gut adapts and develops.

The g-tube. That's a tough one. We really struggled with that decision as waves of memories of Ollie came crashing down with each discussion. In the end, conversations with doctors at both UNC and WakeMed helped convince us it was the best course for Isaak. With a g-tube in place, they can feed him continuously and help encourage bowel development, but without the potential negative assocations that might occur with the use of an OG or NG tube for an extended period. He'll still be able to eat orally, but they can increase the quantity and spread out the feeds using the g-tube. All in all, he should get home earlier this way and have a better outcome to boot.

If we are very lucky, the g-tube combined with oral feeds might mean he can go home sans TPN, but it's not likely. He will most likely be getting a mix of enteral and parenteral nutrition for some time to come. Gradually, his gut should adapt and he'll go off the TPN. This would be good for his liver as extended use of parenteral nutrition places a strain on multiple organs.

Surgery is scheduled between 10 and 1. Really, it's like the cable guy or something. The surgeon has several smaller cases he wants to finish up in the morning before taking on Isaak's. He'll be in good hands.

We will have a single running post tomorrow to keep the world up to speed as we know something. He was sleeping very peacefully this evening so hopefully he gets a good night's rest. Something I'm about to try to do.

Penny is doing well. We are trying to get her to UNC so Bekka can nurse. The more she can do that, the faster Penny will get home. She really is so close. She needs to desat a little less and move to completely oral feeds.

Both of the little guys seem so big now. Isaak cracked 2500g tonight (5.5 lbs) and Penny is just under 2400g (5lbs 5oz). He's 17.5 inches long and she is just over 16.5. It's amazing to look at these chunky little monkeys and think about where we were 73 days ago. We were so worried about getting him to even 1800g a few weeks ago. Those guys at WakeMed did a great job of fattening him up!

8 Weeks, 2 Days and Growing Like Weeds

Well, it's been almost a week since the last post, so I figure the twins' adoring public deserves another update. Overall, it's a happy thing we haven't posted as the news is almost all positive. So the old "no news is good news" adage applies here. Mostly, I figure you want more pictures, and we have those right here!

Isaak finished up his course of Epogen this week, and boy what a difference it made. He had been doing quite well from a respiratory standpoint but began showing signs of breathing difficulties before they started the treatment. He was also pale as a ghost. They didn't want to transfuse because of the various risks associated with the procedure. Almost as importantly, severe anemia tends to jump start red blood cell production and a transfusion can stop that process cold. They began the Epogen course and hoped that it would kick in before a transfusion was absolutely required. It appears to have worked. They won't do another reticulocyte or hematocrit count for a couple of days, but visually, he is like a different baby. He spends most of his time at room air concentrations with about 2 lpm of flow. With luck, they will begin turning down his flow a little bit at a time.

The biggest concern with Isaak has really been his weight gain (or lack thereof). He has been getting a mix of oral and IV nutrition but had nearly stopped gaining weight. You could see that he was longer, but just wasn't getting much heavier. Each time he would gain a few ounces, he'd give back half of that in a couple days. This was likely caused by a combination of the "dumping" he was experiencing from his ostomy and possibly the anemia (the body has to work harder to cover for the low hematocrit). Even after the dumping improved dramatically he still wasn't gaining as quickly as needed to hit his targets in preparation for the surgery on July 13. Remember, they really want him at or as close to 2kg (4lb 6.5oz) as possible. The doctors and NPs spent quite a lot of time brainstorming over the weekend and made several adjustments including lowering his oral intake to allow for increased TPN.

It appears to have worked like a charm.

Little guy has gone from 1500g (3lb 5oz) two days ago to 1720g (3lb 13oz) tonight. His ostomy output is now very, very good. After the first night of gain, we all held our breath as we were afraid it was a fluke, but it's occurred two nights in a row. He's not puffy at all so no one thinks it's just water weight. The TPN is doing it's job right now, but of course, we hope that once his ostomy is repaired, they will be able to dramatically reduce the TPN quantity. There are many complications resulting from extended use of parenteral nutrition. The good news for him is that getting any oral food at all has been shown to have a really good protective effect on the liver and other organs.

Penny's biggest challenge has certainly not been gaining weight. The little piglet (or piglette, as her mama likes to call her) is now getting 38ml of breast milk per feed and weighed in at 1841g (4lbs 1oz) tonight! Her primary issue has been breathing. She'd been outpacing Isaak for so long, but took a slide early in the week. They ended up bumping her flow to 2 lpm (from 1 lpm a couple of weeks ago). The concern was that even at the increased flow rate, she was still needing between 28 and 30% oxygen. That is not a particularly high concentration, but we really want to be weaning them off all respiratory support. The solution was a dose of Lasix. She had begun to look puffy and edema can cause major issues with breathing since the lungs are one of the first places that fluid tends to go. After the dose, she lost almost 100g in water weight and then proceeded to get right back on track with what looks like real, solid gains.

Oh, I almost forgot, if you haven't cheated and looked at the pictures first, Penny is in a big girl crib! No more isolette for her. They began having a hard time keeping her cool enough so early Sunday morning she got a bed change. If Isaak gets a little bigger and demonstrates he can also regulate his body temp, he'll join her soon (literally, the little guys will be hanging out in the same spot for the first time since they were born).

Update on the Babbies

Both of the little monkeys were doing pretty well tonight. Get this: Isaak is now off all breathing assistance. Will this last? There is a good chance it will. He tried to go off a couple of weeks ago but couldn't quite hack it, however, he has been doing fantastically well from a respiratory standpoint and they finally decided to try again.

He's up to 4ml per hour on his feeds and is processing everything well. They are trying to get the right balance of oral feeds and TPN via IV that will help him pack on the ounces but not provide too much fluid. If they give him too much total liquid, they are worried that it could lead to pulmonary edema and we don't want that! If weight gain really becomes a problem, they will have to back down from the oral feeds and increase the IVs. We'd prefer not to do that as having a good balance helps to minimize the risk of complications and liver damage.

Penny weighed in at 1220g tonight and Isaak at 1280g. He's been up and down a bit, so we are hoping he will stay on an upward trend as he really needs to be between 1.8 and 2.0 kilos prior to the ostomy repair surgery. He looks *great* right now, with pink little skin and is just generally really active.

Penny had another pack of HMF added to her feed (that makes 5). She is doing OK from a respiratory aspect but her little brother really ran right past her. It appears that the epo might be kicking in though, as she has been running at 25% O2 the last couple of days (vs. the 30+ she's been running the last two weeks). Little girl decided she didn't want mommy and daddy to leave this evening and had a moderately long but not very deep brady and desat spell. Since the events mentioned in the last update, she's been *much* better overall (no desats to the 20s or neopuffs readied.) If the epo is successful and she continues the steady weight gain, the spells should start disappearing soon.

We'll try to get some pictures up before the end of the weekend.